Congenital pressure necrosis of the forearm in a newborn infant.

The authors report a case of congenital pressure necrosis of the forearm presenting in a newborn infant. The patient presented with an edematous and purpuric upper extremity with no underlying vascular compromise. After demarcation of the nonviable tissue during the first month of life, the extremity was debrided, and a thin split-thickness skin graft was applied. Graft contracture over the subsequent year was released and reconstructed with a full-thickness skin graft. Patient follow-up at age 22 months demonstrated progressive return of strength and function of the extremity with restoration of soft-tissue bulk and contour. When presented with this unusual circumstance, the plastic surgeon should be familiar with its differential diagnosis and management. Conservative debridement and age-appropriate resurfacing of the remaining wound were the essential treatment principles followed in this patient.

Pediatric dermatology.

The latest studies regarding the controversial and concerning subjects of pigmented nevi and melanoma in children are reviewed. Additional topics covered include dermatologic conditions that feature genetic mosaicism, theories of pathogenesis of Langerhans cell histiocytosis, new clinical information regarding juvenile xanthogranulomas, and the unique features of pyoderma gangrenosum in infants and children.

Cutaneous telangiectases in neonatal lupus erythematosus.

BACKGROUND: Persistent telangiectases are a feature of neonatal lupus erythematosus (NLE) but have generally been noted in areas of prior inflammatory disease. The occurrence of vascular macules and papules at sites without preceding dermatitis has not been reported in NLE. OBJECTIVE: Our purpose was to emphasize a previously unidentified aspect of cutaneous NLE: the presence of angiomatous or matlike telangiectases in sites without antecedent or concurrent dermatitis. METHODS: We describe seven patients in which telangiectatic macules or angiomatous papules were primary or early features of NLE. RESULTS: Five infants lacked a history of preceding or concurrent inflammatory lesions at sites of telangiectasia development. In four infants findings included vulvar or inguinal angiomatous papules. CONCLUSION: Telangiectases may be a presenting feature of NLE, can be found in sun-protected sites, and may occur independent of "lupus dermatitis."

Recurrent palmoplantar hidradenitis in children.

BACKGROUND: There are several reports of tender, erythematous plantar nodules occurring in pediatric patients. Despite similar morphological features, the histologic findings in these lesions have been quite diverse. A new entity called idiopathic plantar hidradenitis (also termed neutrophilic eccrine hidradenitis in children), which is characterized by tender, red nodules on the feet and unique histopathologic features, was recently described. OBSERVATIONS: We describe two children with these unusual cutaneous lesions and histopathologic findings and compare them with patients with idiopathic plantar hidradenitis. Our patients, in contrast to those with idiopathic plantar hidradenitis, had involvement of the palms as well as the soles. Both children had self-limited recurrent lesions; in one child, the lesions were associated with low-grade fever. Biopsy specimen findings in both cases demonstrated dense neutrophilic infiltrates localized to the eccrine units. CONCLUSIONS: Our patients had clinical and histologic findings similar to those recently reported as idiopathic plantar hidradenitis (neutrophilic eccrine hidradenitis in children). Because palms and soles can be affected and the lesions typically recur, we suggest that this condition be referred to as recurrent palmoplantar hidradenitis.

The gene for Bazex-Dupré-Christol syndrome maps to chromosome Xq.

Bazex-Dupré-Christol syndrome is an inherited condition with skin cancer predisposition characterized by follicular atrophoderma, hypotrichosis, and early onset of multiple basal cell carcinomas. Previous reports suggested an X-linked mode of inheritance. We therefore performed linkage analysis with microsatellite markers of the X chromosome in three families. We obtained evidence for X-linkage and regional assignment to Xq24-q27 of this syndrome (maximal lod score = 5.26 with a recombination fraction of 0% at the DXS1192 locus). This represents a first step towards the identification of a gene involved in hair follicle development and skin tumor formation.

Nevoid basal cell carcinoma syndrome. Report of a case with associated Hodgkin's disease.

PURPOSE: Nevoid basal cell carcinoma syndrome (NBCCS) is an autosomal-dominant multisystem disorder characterized by various congenital anomalies, multiple cutaneous basal cell carcinomas (BCCs), and increased risk for other benign and malignant tumors. We report a 13-year-old girl with NBCCS who developed cutaneous BCCs 2 years after radiotherapy for Hodgkin's lymphoma. PATIENTS AND METHODS: Review of clinical and laboratory observations of patients with NBCCS suggest a close relationship between radiotherapy and the early emergence of numerous BCCs. However, in vitro studies of the effect of radiosensitivity on cells cultured from patients with NBCCS show inconsistent results. RESULTS: Clarification of the role of radiotherapy in the induction of BCCs in NBCCS requires additional studies. Nevertheless, recognition of the syndrome is important because whenever possible radiotherapy for secondary tumors might be effectively replaced by chemotherapy.

Painful keratoderma and photophobia: hallmarks of tyrosinemia type II.

Tyrosinemia type II (Richner-Hanhart syndrome), which is caused by a deficiency of hepatic tyrosine aminotransferase, results in elevated plasma and urinary tyrosine concentrations. We describe a young boy who was seen at 6 months of age with red eyes, photophobia, and eye pain that were not suspected to be caused by tyrosinemia II until painful plantar keratoderma developed at 2 1/2 years of age. Treatment with a diet low in tyrosine and phenylalanine reversed the manifestations of the disease.

Streptococcal toxic shock-like syndrome.

BACKGROUND: After decades marked by a decrease in the incidence and severity of scarlet fever and streptococcal soft-tissue infections, invasive infections with group A streptococci have reemerged as a global public health problem. Sporadic outbreaks of a rapidly progressive disorder characterized by fever, shock, desquamating rash, and multiorgan system failure often associated with severe suppurative soft-tissue infection have recently been recognized in young, otherwise-healthy adults. Referred to as streptococcal toxic shock-like syndrome, this acute, progressive, often fatal illness appears to be related to an overall change in the virulence of group A streptococci and the reappearance of highly mucoid exotoxin-producing strains. However, the relationship between virulence factors, epidemiological features, and precise pathogenesis of these infections remains unclear. OBSERVATIONS: The 80% incidence of soft-tissue involvement in streptococcal toxic shock-like syndrome and the high frequency of progression to necrotizing fasciitis, myositis, or even death are unprecedented. The type and extent of tissue injury, systemic toxicity, and multiorgan system failure associated with this syndrome appear to be mediated by streptococcal pyrogenic exotoxins via the induction of biologically potent endogenous cytokines. The observed increase in the incidence and severity of invasive group A streptococcal infections coincides with the resurgence of genetically related, highly virulent strains of Staphylococcus pyogenes that express an invasive phenotype and carry a specific gene for pyrogenic exotoxin A. CONCLUSIONS: The changing spectrum of invasive group A streptococcal infections has prompted a reexamination of the microorganism and its diverse clinical manifestations. A clonal basis for increased virulence expression is supported epidemiologically. As the incidence of streptococcal toxic shock-like syndrome appears to be increasing, it is imperative that clinicians become familiar with the presentation of this syndrome so they can diagnose and treat it in a timely and effective manner.

An interdisciplinary group for parents of children with hemangiomas.

Parents are shocked by the unexpected appearance and growth of hemangiomas, the most common benign tumors of childhood, yet parents' psychosocial needs are largely ignored. A psychoeducational/self-help pilot group for parents is described in this article. The Mental Health Inventory and a 44-item questionnaire were used to gather pertinent biopsychosocial data. Parents were more distressed and had less of a sense of well-being than the general population. Medical complications were significantly associated with the couple's distress (P < 0.05). Parental perception of hemangiomal surface area or site was not correlated with parental distress. Therapists clinically assessed parental distress, presented coping strategies for parents and families, and discussed medical information about hemangiomas and normal childhood development.

Acrally occurring dermatofibrosarcoma protuberans in children and adults.

BACKGROUND: Dermatofibrosarcoma protuberans (DFSP) arising on the hands and feet is unusual and, at any site, is extremely rare in children. OBJECTIVE: We report a 5-year-old girl who developed DFSP on her hand at 9 months of age. We review the clinical features and treatment of childhood DFSP and of acrally occurring DFSP at all ages. CONCLUSION: DFSP in children develops most often on the back (22.2%) and at acral sites (14.8%). It occurs on the hands and feet of children more commonly than in adults. This may be related to the increased frequency of trauma at these sites in children. Mohs micrographic surgery is the treatment of choice for DFSP.

Cutaneous mucinosis of infancy.

Cutaneous mucinosis of infancy has been reported only rarely in the literature. We describe a case occurring in a black infant girl. Although no associated abnormalities have been described previously, our patient had a history of developmental delay, congenital cataracts, bilateral inguinal hernias, and an accessory tragus. The significance of these features is unclear.

Atopic dermatitis and ichthyosis vulgaris.

Atopic dermatitis remains a common skin problem in the pediatric age group. General approaches to management focus on reducing inflammation and pruritus as well as preventing xerosis. Ichthyosis vulgaris is the most common form of the ichthyoses and often is associated with atopic dermatitis. Recognition of these conditions is necessary to institute therapy that will alleviate the discomfort experienced by affected individuals.

Cutaneous porphyrialike photosensitivity after liver transplantation.

BACKGROUND: Liver disease and cholestasis are often associated with abnormal coproporphyrin levels, but photosensitivity eruptions do not usually occur with these conditions. We present a case of a cutaneous porphyrialike photosensitivity in a liver transplant patient with only mildly elevated coproporphyrin levels. OBSERVATIONS: A 5-year-old girl developed a cutaneous porphyria cutanea tarda-like photosensitivity after liver transplantation for congenital biliary atresia. Her cutaneous eruption consisted of fluid-filled vesicles and crusted erosions involving her face and arms. These lesions eventually healed with atrophic scarring and milia. The patient was in a state of chronic hepatic rejection, but her serum and 24-hour urine specimens contained only mildly elevated levels of coproporphyrin. Results of histopathologic examination and direct immunofluorescence of a skin biopsy specimen resembled those of porphyria. CONCLUSIONS: Although clinically porphyria cutanea tarda was suspected, her porphyrin profile was not consistent with that diagnosis. Liver transplantation has become relatively common, but porphyria cutanea tarda-like clinical and histopathologic findings associated with coproporphyrinemia in a liver transplant patient have not been reported to date.

Pyoderma gangrenosum in infants and children.

Pyoderma gangrenosum is an uncommon ulcerative skin disorder that occurs in all age groups. Approximately 4% of patients are infants and children. There are several notable differences between the childhood and adult manifestations of the disease, including the distribution of lesions and associated disorders. We reviewed the childhood cases (< or = 18 yrs of age) of unequivocal pyoderma gangrenosum in the English literature and tabulated the trends in clinical features, associated disorders, and therapy. We report our 3-week-old patient, the youngest documented case. Of the 46 patients, only 4 were less than 1 year of age. A systemic illness was present in 74% of the older children, most commonly, ulcerative colitis. Only one infant had an associated problem (HIV+) at the time of onset. Infants appear to have an unusual distribution of perianal and genital lesions not often described in other age groups. Our review suggests that pyoderma gangrenosum in children has a similar clinical appearance to that in adults. It is associated with some of the same underlying disorders, but with different frequencies. The distribution of lesions in children is similar, often involving the lower extremities, but pyoderma gangrenosum of the head and face appears to be more common in children. Infants may have ulcers in genital and perianal areas. The most frequently prescribed treatment for children is systemic corticosteroids, which generally are very effective.

Inflammatory bullous diseases in children.

Bullous disease in children can be confusing clinically and challenging to mange. Five groups of autoimmune bullous disease are discussed with emphasis on unique features in children. Pemphigus (vulgaris, foliaceus, neonatal), pemphigoid (bullous and cicatricial), dermatitis herpetiformis, linear IgA dermatosis, and epidermolysis bullosa acquisita are uncommon pediatric disorders. A high index of suspicion is necessary to diagnose and treat these conditions without delay.

Antimalarials for children: indications, toxicities, and guidelines.

The use of antimalarial drugs in children has been discussed extensively in the rheumatology literature, but there is scant information in the dermatology literature. We discuss indications, dosing, administration, and side effects of antimalarial drugs in children as well as skin diseases in children who have been treated with chloroquine or hydroxychloroquine.